What Is Tricuspid Atresia?

Your heart is made up of four chambers. Heart valves between the chambers control the flow of blood in the heart. These valves open and close when your heart contracts. The four valves of the heart include:

the tricuspid valve

the pulmonary valve

the mitral valve

the aortic valve

In some newborns, the tricuspid valve, which is located on the right side of the heart, isn’t present or has not properly developed. The absence of the tricuspid valve in newborns is known as tricuspid atresia. Tricuspid atresia is a rare congenital (present at birth) heart disorder. It is a serious disorder, and can be life threatening.

Types of Tricuspid Atresia

Although the absence of the tricuspid valve itself is diagnosed as tricuspid atresia, there are several different outcomes that can result from this absence.

In some cases, the absence of the valve will produce a thick wall of tissue that completely blocks the flow of blood in the right chambers of the heart. When this happens the blood cannot flow and the right side of the heart may be small and underdeveloped.

In some newborns, a hole known as a ventricular septal defect (VSD) is present where the tricuspid valve should be. This hole will make it difficult for the heart to pump blood to the lungs to pick up oxygen. There are three forms of this disorder that are classified by the size of the hole in the heart:

Large VSD—If the hole in the heart is large, too much blood may be pumped to the lungs. This can result in a condition known as congestive heart failure (CHF).

Moderate VSD—In this condition the VSD is medium-sized, allowing some blood to be pumped to the lungs. Newborns with this condition will experience less distress than those with large or small VSDs.

Small VSD—If the VSD is small, not enough blood will be pumped to the lungs to pick up oxygen. A lack of oxygen to vital organs will cause the newborn’s skin to turn blue (cyanosis).

Tricuspid atresia may also be accompanied by a condition known as transposition of the great arteries (TGA). In this condition the major vessels of the heart—the aorta and pulmonary artery—are reversed.

What Causes Tricuspid Atresia?

The heart begins to form when a fetus is just 8 weeks old. During this period of growth, failure of the tricuspid valve to develop will result in tricuspid atresia. Doctors do not know what causes this to occur. Tricuspid atresia is often common in families (hereditary) and is associated with certain genetic or chromosomal abnormalities including Down syndrome.

What Are the Symptoms of Tricuspid Atresia?

The symptoms of tricuspid atresia that develop will depend on the specific heart defect that is present. Babies that are born with tricuspid atresia typically show signs of distress within the first few hours of life. However, some babies may be healthy at birth, and only begin to show symptoms in the first two months of life.

Common symptoms associated with tricuspid atresia include:

rapid breathing

shortness of breath (dyspnea)

blue skin color (cyanosis)

fatigue that occurs during feeding

slow growth

sweating

If the hole in the newborn’s heart is big enough to cause congestive heart failure, there may be other symptoms associated with this condition. These include coughing and swelling in the legs and feet (a syndrome called “edema”).

How Is Tricuspid Atresia Diagnosed?

Tricuspid atresia may be identified by your doctor while your baby is still in the womb. Routine prenatal ultrasounds used to monitor the growth of your baby may detect abnormalities in heart of the fetus.

After your baby is born, your doctor will be able to diagnose tricuspid atresia by performing a physical exam. Your doctor may detect a heart murmur and may notice a bluish tone to your baby’s skin. Your doctor may also perform several tests to confirm the diagnosis. Tests may include:

ultrasound of the heart

electrocardiogram (ECG)

echocardiogram

chest X-ray

cardiac catheterization

MRI of the heart

How Is Tricuspid Atresia Treated?

If your baby is diagnosed with tricuspid atresia, he or she will be admitted to a neonatal intensive care unit (NICU). Here your baby will receive specialized care to treat the condition. If your baby cannot breathe, he or she may be put on a breathing machine (ventilator). Your baby may also be given medication to maintain heart function.

After your baby is stabilized, surgery will be required to correct the heart defect. For infants that have tricuspid atresia, three surgeries are typically needed as the child’s heart and body grow and change. In most infants with this condition, the first heart surgery is performed in the first few days of life. This procedure involves placing a shunt in the heart to keep blood flowing to the lungs.

Following the surgery, your baby will be discharged and sent home. He or she will require medications and monitoring to detect complications. Your baby’s care will be overseen by a pediatric cardiologist. This doctor will determine when your baby is ready for a second surgery.

The second surgery is typically performed when your baby is between 3 and 6 months old. This operation is known as the Glenn shunt or hemi-Fontan procedure. Following this surgery, your baby will require one more procedure that will take place when your baby is between 18 months and 5 years of age. This procedure is known as the Fontan procedure. In this final surgery, your child’s surgeon will create a path for oxygen-depleted blood returning to the heart to flow into the arteries that will take it directly to the lungs.

In the rare cases where your child is not a candidate for these surgical procedures, doctors will discuss the possibility of a heart transplant (Mayo, 2011).

What Is the Outlook for an Infant With Tricuspid Atresia?

An infant diagnosed with tricuspid atresia will require continued care for years after successful treatment. Open heart surgery will be required and this surgery carries with it a number of potential complications, including heart failure.

After all of the surgeries to correct tricuspid atresia are completed, your baby will require ongoing care and monitoring into adulthood. Children with tricuspid atresia will be at increased risk for developing stroke, congestive heart failure, infections, and arrhythmia across their lifespan. Medications may be needed to help prevent these conditions.